Amelogenesis imperfecta (AI) is a disorder group of hereditary development that affects the dental enamel structure in the primary and permanent teeth. The enamel may be hypoplastic, hypomineralised, hypomature or both and teeth affected may be discolored, sensitive or prone to disintegration. AI exists in isolation or associated with other abnormalities in syndromes. It may show autosomal dominant, autosomal recessive, sex-linked and sporadic inheritance patterns. Clinical treatment is important to address the esthetic appearance of affected teeth, reduce dentinal sensitivity, preserve tooth structure, and optimize masticatory function. The purpose of this paper was to describe the diagnosis, treatment planning, and dental rehabilitation of a girl with autosomal recessive amelogenesis imperfecta. Through this observation, we conclude that the Rehabilitation of a patient with amelogenesis imperfecta (AI) from both the functional and esthetic standpoints represents a challenge. The complexity of the condition requires an interdisciplinary approach for optimal treatment outcomes.